The occurrence of cystic fibrosis and celiac sprue within a single sibship.
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Biomedical subjects
Publications and source records attributed to P Asquith.
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Lymphocytes and plasma cells in the lamina propria and lymphocyte in the epithelium have been quantitated in jejunal biopsies from 18 patients with coeliac disease who developed lymphoma. In six patients two or more serial biopsies were available for study. Counts were compared with those obtained in 15 healthy controls and 30 other coeliacs, 15 of whom have been on a gluten-free diet for more than 12 months. Results showed that although the lymphoma patients have abnormal counts compared with controls, they differed from untreated coeliacs in that they had lower plasma cell and higher lymphocyte counts in the lamina propria and lower lymphocyte counts in the epithelium. Such changes tended to be present for up to five years before the diagnosis of lymphoma was made. The results suggest that the immunological status of coeliac patients with lymphoma differs from that of other coeliacs and it could represent a primary abnormality and also be relevant to the development of lymphoma.
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A comparison has been made of inflammatory cell counts in the lamina propria and epithelium of jejunal biopsies in 11 patients with adult coeliac disease with those found in 12 control subjects. In the coeliac patients, there were significant increases in the numbers of total cells, plasma cells, and intraepithelial lymphocytes, but a significant reduction in lamina propria lymphocytes. Following clinical improvement on a strict gluten-free diet, significant changes in cell counts occurred, but with the exception of lymphocytes in the lamina propria, the counts were still abnormal. Analysis of five patients in whom the biopsy improved to near normal morphology and of six in whom there was no such improvement showed that significant falls in plasma cells and rises in lymphocytes in the lamina propria could occur without improvement in other morphological appearances. These results seem relevant to the problem of diagnosing coeliac disease in patients who, on gluten withdrawal, show an unequivocal clinical response, but no gross morphological improvement in the jejunal biopsy. On the basis of the observed changes in cell counts, there seems little justification in questioning the diagnosis of coeliac disease in such patients.
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Among 569 schoolchildren (386 boys and 183 girls) aged 14-17 years, 233 had serum alkaline phosphatase values of 30 K.A. units or greater. There was no significant difference in the results in Asian, white, or West Indian children. The mean values were significantly greater in boys than girls and both showed a fall in mean values with increasing age. Radiological rickets occurred in at least 4% of the survey, and was more common in Asians. Low calcium and high hydroxyproline excretion in most of those investigated and the response to vitamin D therapy suggests that most children with alkaline phosphatase levels above 30 K.A. units have rickets.Since the decline of the widespread supplementation of the diet with vitamin D, the demands of the physiological growth spurt for extra vitamin D in adolescents already on a borderline intake may be responsible for the great increase in "biochemical" rickets. Once the growth spurt is over the condition subsides but the results of impaired growth or permanent pelvic deformity will not necessarily be eradicated.
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In a study of histocompatibility antigens in adult coeliac disease the HL-A phenotypes have been determined in 117 patients and 149 of their first-degree relatives, of whom 94 had had a jejunal biopsy performed. There was an increased frequency of the HL-A 8 antigen in the patients and their relatives. Family studies showed no irregularity in the inheritance of both this antigen and the HL-A 1 and 8 haplotype.
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